ERJ Open Research最新文献

{"title":"Williams-Campbell syndrome case series and discordant twins.","authors":"Laura Sellmer, Judith Spiro, James Chalmers, Stefano Aliberti, Eva Polverino, Pontus Mertsch","doi":"10.1183/23120541.00219-2024","DOIUrl":"10.1183/23120541.00219-2024","url":null,"abstract":"<p><p><b>Williams-Campbell syndrome (WCS) presents diagnostic challenges. This case series highlights clinical complexities and genetic/environmental interplay, and underscores the need for personalised treatment approaches. #WCS #RareDisease</b> https://bit.ly/3Xqze8x.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11514199/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521414","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Decoding the impact of the placebo response in clinical trials for chronic cough.","authors":"Mengru Zhang, Bangyu Zhang, Alyn H Morice","doi":"10.1183/23120541.00335-2024","DOIUrl":"10.1183/23120541.00335-2024","url":null,"abstract":"<p><p>Chronic cough is a prevalent and challenging condition, with limited treatment options available. The interpretation of clinical trial results for antitussive drugs is complicated by the presence of the placebo response, which can confound outcomes and impede regulatory approval. This review aims to explore the impact of the placebo response on clinical trials for cough medications and elucidate the underlying mechanisms involved. The multifaceted nature of antitussive effects, including pharmacological, psychological/neurobiological and nonspecific effects, is discussed. Additionally, potential solutions to address the placebo response in future cough medication development, such as strategic study design, appropriate choice of end-points and meticulous patient selection, are proposed. More progress to harness this issue is urgently needed.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11514000/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521410","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effects of acetazolamide on sleep disordered breathing in pulmonary vascular disease: a randomised controlled trial.","authors":"Esther I Schwarz, Stéphanie Saxer, Mona Lichtblau, Simon R Schneider, Julian Müller, Laura Mayer, Konrad E Bloch, Silvia Ulrich","doi":"10.1183/23120541.00040-2024","DOIUrl":"10.1183/23120541.00040-2024","url":null,"abstract":"<p><strong>Background: </strong>Patients with pulmonary vascular disease (PVD) often suffer from nocturnal hypoxaemia, but also from sleep apnoea. Short-term use of acetazolamide increases ventilation due to metabolic acidosis and also reduces loop gain. We investigated whether prolonged use of acetazolamide improves sleep disordered breathing in PVD.</p><p><strong>Methods: </strong>In a randomised controlled crossover trial, patients with PVD were randomly assigned to acetazolamide 250 mg and placebo twice daily for 5 weeks. Patients underwent respiratory polygraphy at baseline and at the end of each intervention phase. Outcomes of interest were the effect of acetazolamide on mean nocturnal oxygen saturation (<i>S</i> _pO₂ ), time with oxygen saturation <90% (<i>t</i> _<90), apnoea-hypopnoea index (AHI) and sleep apnoea severity.</p><p><strong>Results: </strong>In 20 patients with PVD (55% women, nine with pulmonary arterial hypertension, 11 with distal chronic thromboembolic pulmonary hypertension; mean±sd nocturnal <i>S</i> _pO₂ 88.8±3.5%, obstructive AHI 12.6±12.3 events·h^-1), 5 weeks of acetazolamide resulted in a significant improvement in nocturnal oxygenation compared to placebo (mean nocturnal <i>S</i> _pO₂ +2.3% (95% CI 1.3-3.3%); p<0.001 and <i>t</i> _<90 -18.8% (95% CI -29.6- -8.0%); p=0.001). Acetazolamide increased the proportion of patients with mean nocturnal <i>S</i> _pO₂ ≥90% from 45% to 85%. The percentage of patients with AHI >5 events·h^-1 was reduced from 75% to 60% and with AHI >15 events·h^-1 from 30% to 15%. Two patients discontinued the study because of mild side-effects.</p><p><strong>Conclusions: </strong>Acetazolamide given for 5 weeks reduces nocturnal hypoxaemia in PVD to a clinically relevant level and reduces the proportion of patients with obstructive sleep apnoea.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11514193/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521411","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term functional course of Sjögren's disease-associated interstitial lung disease.","authors":"Caroline Diou, Marie-Pierre Debray, Raphaël Porcher, Catherine Bancal, Karime Sacre, Camille Taille, Warda Khamis, Robin Dhote, Raphaël Borie, Hilario Nunes, Yurdagül Uzunhan, Bruno Crestani","doi":"10.1183/23120541.00384-2024","DOIUrl":"10.1183/23120541.00384-2024","url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung disease (ILD) is common in primary Sjögren's disease (pSD); its functional course is poorly known. Our aim was to characterise the long-term functional course and prognosis in patients with pSD-ILD. We determined the role of baseline demographic and clinical variables in the evolution of lung function and identified risk factors for death or transplantation.</p><p><strong>Methods: </strong>In a retrospective observational cohort study, patients with pSD and ILD were retrospectively identified from two French ILD centres. Forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (<i>D</i> _LCO) slopes were obtained from joint models. Latent class mixed models identified clusters of FVC and <i>D</i> _LCO trajectories.</p><p><strong>Results: </strong>We included 73 patients (63% women, mean age 63 years), with a median follow-up of 9.3 years. At baseline, mean FVC was 73±21% and <i>D</i> _LCO 51±16%. On average, FVC was stable, while there was an annual decline in <i>D</i> _LCO of 1% of the predicted value. Male sex, a pattern of usual interstitial pneumonia (UIP) or indeterminate for UIP on high-resolution computed tomography (HRCT), and features of fibrosis on HRCT, were associated with an accelerated decline in FVC and <i>D</i> _LCO.</p><p><strong>Conclusion: </strong>We identified clusters of lung function evolution. 1) Two FVC trajectories: patients with stable FVC (n=56, 78%); patients with FVC decline (n=16, 22%) of 2.4% per year, characterised by a low baseline <i>D</i> _LCO (39%) and a higher risk of death or transplantation (HR 52, 95% CI 10-273). 2) Three <i>D</i> _LCO trajectories: patients with stable <i>D</i> _LCO (n=44, 66%); patients with a slow decline in <i>D</i> _LCO (n=12, 18%) of 2.8% per year; patients with a rapid decline in <i>D</i> _LCO (n=11, 16%) of 4.8% per year, characterised by a low baseline <i>D</i> _LCO (41%) and a higher risk of death or transplantation (HR 156, 95% CI 18-1352).</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11513999/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521412","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Altered ventricular repolarisation dynamic: the missing link between obstructive sleep apnoea and sudden death?","authors":"Karim Benali, Kanchan Kulkarni, Frederic Roche","doi":"10.1183/23120541.00604-2024","DOIUrl":"10.1183/23120541.00604-2024","url":null,"abstract":"<p><p><b>Research is needed to explore the broader links between oxygen desaturation episodes, ventricular repolarisation instability and genesis of malignant arrhythmic events</b> https://bit.ly/3WeQNHy.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11513998/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142521409","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cognitive interference of respiratory <i>versus</i> limb muscle dual tasking in healthy adults.","authors":"Peter Rassam, Tamires de Mori, Marine Van Hollebeke, Dmitry Rozenberg, Paul Davenport, Lori Ann Vallis, W Darlene Reid","doi":"10.1183/23120541.00169-2024","DOIUrl":"https://doi.org/10.1183/23120541.00169-2024","url":null,"abstract":"<p><strong>Background: </strong>Inspiratory threshold loading (ITL) and associated dyspnoea have been shown to interfere with cognition during cognitive-motor dual tasking. However, ITL has not been compared with another rhythmic muscle activity, such as lower limb pedalling. While ITL has been shown to interfere with cognition, the mechanism of the prefrontal cortex (PFC) during ITL or other rhythmical muscle dual tasking, has not been elucidated. Given the cognitive interference that arises during ITL, we hypothesise that ITL cognitive-motor dual tasking will result in greater cognitive decrements and increased PFC activity compared with the pedalling cognitive-motor dual task.</p><p><strong>Methods: </strong>30 healthy participants (16 females; median age 23 (interquartile range 23-24) years) were recruited. They performed five 3-min tasks in a single visit in a random order: single tasks were ITL, pedalling and Stroop task and dual tasks were ITL-Stroop and pedalling-Stroop. Participant's PFC activity was assessed bilaterally using functional near-infrared spectroscopy throughout each task. Single- and dual-task cognitive performance was evaluated by measuring Stroop task reaction time and accuracy. Dyspnoea and rating of perceived exertion were evaluated at the end of each task.</p><p><strong>Results: </strong>ITL-Stroop resulted in greater impairments in reaction time (p<0.001), accuracy (p<0.01) and increased medial/dorsolateral PFC activity (p≤0.006) than pedalling-Stroop. ITL-Stroop elicited greater Borg dyspnoea and rating of perceived exertion than pedalling-Stroop (p<0.001), despite pedalling-Stroop having a greater heart rate response (p<0.001).</p><p><strong>Conclusion: </strong>The heightened cognitive decrements, perceptual response and PFC activity suggest that inspiratory muscle loading and its accompanied dyspnoea results in greater cognitive interference than rhythmic pedalling.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11456971/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142389016","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"High-flow humidified oxygen as an early intervention in children with acute severe asthma: a feasibility randomised controlled trial.","authors":"Akshat Kapur, Héctor Rojas-Anaya, Graham Roberts, Damian Roland, Atul Gupta, Michaela Lazner, Jane Bayreuther, Fleur Cantle, Christina Jones, John Pappachan, Stephen Bremner, David James, Shane Fitzgerald, Kelly Owens, Lalarukh Asim, Ekaterina Khaleva, Paul Seddon","doi":"10.1183/23120541.00168-2024","DOIUrl":"https://doi.org/10.1183/23120541.00168-2024","url":null,"abstract":"<p><strong>Background: </strong>Treating children with acute severe asthma (ASA) who fail to respond to first-line inhaled bronchodilators is problematic: use of intravenous agents is inconsistent and side-effects are common. High-flow humidified oxygen (HiFlo) has shown promise in other respiratory conditions and is increasingly used in ASA, but with little evidence.</p><p><strong>Methods: </strong>We conducted a feasibility randomised controlled trial with deferred consent to assess early HiFlo in children aged 2-11 years with ASA not responding to \"burst\" therapy (high-dose inhaled salbutamol ± ipratropium). Children with Paediatric Respiratory Assessment Measure (PRAM) score 5+ after \"burst\" were randomised to commence HiFlo or follow standard care. Candidate primary outcomes assessed were treatment failure requiring escalation, and time to meeting hospital discharge criteria.</p><p><strong>Results: </strong>The target was met despite coronavirus disease 2019 pandemic disruption: 56 children were randomised across four sites, with deferred consent received in 50 out of 56 (89%), and mean recruitment rate 1.1 per site per month. 28 were allocated early HiFlo and 22 standard care. Data collection was complete for both candidate primary outcomes. Treatment failure requiring escalation occurred in 18 of 28 children (64%) in the HiFlo arm and in 19 of 22 (86%) in the standard care arm. Median (interquartile range) time from randomisation to meeting discharge criteria was 29.3 h (21.8-43.7 h) in the HiFlo arm and 36.8 h (24.1-46.3 h) in the standard care arm.</p><p><strong>Conclusions: </strong>HiFlo in childhood ASA is a potentially promising intervention whose use is increasing despite lack of evidence. A definitive randomised controlled trial to assess its effectiveness is required and appears to be feasible.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11456965/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142389019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessing post-COVID-19 respiratory dynamics: a comprehensive analysis of pulmonary function, bronchial hyperresponsiveness and bronchodilator response.","authors":"Chun-Yao Huang, Yao-Kuang Wu, Mei-Chen Yang, Kuo-Liang Huang, Wen-Lin Su, Yi-Chih Huang, Wu Chih-Wei, I-Shiang Tzeng, Chou-Chin Lan","doi":"10.1183/23120541.00149-2024","DOIUrl":"https://doi.org/10.1183/23120541.00149-2024","url":null,"abstract":"<p><strong>Background: </strong>Coronavirus disease 2019 (COVID-19) has a considerable impact on the global healthcare system. Individuals who have recovered from COVID often experience chronic respiratory symptoms that affect their daily lives. This study aimed to assess respiratory dynamics such as airway hyperresponsiveness (AHR) and bronchodilator response in post-COVID patients.</p><p><strong>Methods: </strong>This study included 282 adults with respiratory symptoms who underwent provocation tests. The demographic details, clinical symptoms and medical histories were recorded. Baseline spirometry, methacholine challenge tests (MCT) and post-bronchodilator spirometry were performed. Patients were divided into the following four groups: Group 1: non-COVID-19 and negative MCT; Group 2: post-COVID-19 and negative MCT; Group 3: non-COVID-19 and positive MCT; and Group 4: post-COVID-19 and positive MCT.</p><p><strong>Results: </strong>Most post-COVID-19 patients (43.7%) experienced AHR, and wheezing was more common. Patients in Group 4 exhibited increased intensities of dyspnoea, cough and wheezing with the lowest pulmonary function test (PFT) parameters at baseline. Moreover, significant decreases in PFT parameters after the MCT were observed in these patients. Although the prevalence of a low forced expiratory volume in 1 s to forced vital capacity ratio (<70%) was initially 2% in Group 4, it increased to 29% after MCT. No significant differences in allergic history or underlying diseases were observed between the groups.</p><p><strong>Conclusions: </strong>These findings provide comprehensive insights into the AHR and respiratory symptoms of post-COVID-19 individuals, highlighting the characteristics and potential exacerbations in patients with positive MCT results. This emphasises the need of MCT to address respiratory dynamics in post-COVID-19 individuals.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11456966/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142389015","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Corticosteroid therapy in fibrotic interstitial lung disease: a modified Delphi survey.","authors":"Manuela Funke-Chambour, Philipp Suter, Gisli R Jenkins, Leticia Kawano-Dourado, Christopher J Ryerson, Athol U Wells, Michael Kreuter, Kerri A Johannson","doi":"10.1183/23120541.00561-2024","DOIUrl":"https://doi.org/10.1183/23120541.00561-2024","url":null,"abstract":"<p><p><b>The use of steroids in fibrotic interstitial lung diseases is founded on limited evidence. This modified Delphi survey sheds light on current clinical practices. Given the risks of steroids, clinical trials are needed to evaluate efficacy and harm.</b> https://bit.ly/3VkgvbS.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11456968/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142389017","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development and validation of a machine learning-based model for post-sepsis frailty.","authors":"Hye Ju Yeo, Dasom Noh, Tae Hwa Kim, Jin Ho Jang, Young Seok Lee, Sunghoon Park, Jae Young Moon, Kyeongman Jeon, Dong Kyu Oh, Su Yeon Lee, Mi Hyeon Park, Chae-Man Lim, Woo Hyun Cho, Sunyoung Kwon","doi":"10.1183/23120541.00166-2024","DOIUrl":"https://doi.org/10.1183/23120541.00166-2024","url":null,"abstract":"<p><strong>Background: </strong>The development of post-sepsis frailty is a common and significant problem, but it is a challenge to predict.</p><p><strong>Methods: </strong>Data for deep learning were extracted from a national multicentre prospective observational cohort of patients with sepsis in Korea between September 2019 and December 2021. The primary outcome was frailty at survival discharge, defined as a clinical frailty score on the Clinical Frailty Scale ≥5. We developed a deep learning model for predicting frailty after sepsis by 10 variables routinely collected at the recognition of sepsis. With cross-validation, we trained and tuned six machine learning models, including four conventional and two neural network models. Moreover, we computed the importance of each predictor variable in the model. We measured the performance of these models using a temporal validation data set.</p><p><strong>Results: </strong>A total of 8518 patients were included in the analysis; 5463 (64.1%) were frail, and 3055 (35.9%) were non-frail at discharge. The Extreme Gradient Boosting (XGB) achieved the highest area under the receiver operating characteristic curve (AUC) (0.8175) and accuracy (0.7414). To confirm the generalisation performance of artificial intelligence in predicting frailty at discharge, we conducted external validation with the COVID-19 data set. The XGB still showed a good performance with an AUC of 0.7668. The machine learning model could predict frailty despite the disparity in data distribution.</p><p><strong>Conclusion: </strong>The machine learning-based model developed for predicting frailty after sepsis achieved high performance with limited baseline clinical parameters.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"10 5","pages":""},"PeriodicalIF":4.3,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11456972/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142389018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}